Pediatric Radiology

, Volume 39, Supplement 1, pp 27–31 | Cite as

Radiation-sensitive genetically susceptible pediatric sub-populations

  • Ruth A. KleinermanEmail author


Major advances in pediatric cancer treatment have resulted in substantial improvements in survival. However, concern has emerged about the late effects of cancer therapy, especially radiation-related second cancers. Studies of childhood cancer patients with inherited cancer syndromes can provide insights into the interaction between radiation and genetic susceptibility to multiple cancers. Children with retinoblastoma (Rb), neurofibromatosis type 1 (NF1), Li-Fraumeni syndrome (LFS), and nevoid basal cell carcinoma syndrome (NBCCS) are at substantial risk of developing radiation-related second and third cancers. A radiation dose-response for bone and soft-tissue sarcomas has been observed in hereditary Rb patients, with many of these cancers occurring in the radiation field. Studies of NF1 patients irradiated for optic pathway gliomas have reported increased risks of developing another cancer associated with radiotherapy. High relative risks for second and third cancers were observed for a cohort of 200 LFS family members, especially children, possibly related to radiotherapy. Children with NBCCS are very sensitive to radiation and develop multiple basal cell cancers in irradiated areas. Clinicians following these patients should be aware of their increased genetic susceptibility to multiple primary malignancies enhanced by sensitivity to ionizing radiation.


Second cancers Radiotherapy Retinoblastoma Neurofibromatosis type 1 Li-Fraumeni syndrome Nevoid basal cell carcinoma syndrome 



This research was supported by the Intramural Research Program of the National Institutes of Health, National Cancer Institute, Division of Cancer Epidemiology and Genetics.


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Copyright information

© Springer-Verlag 2008

Authors and Affiliations

  1. 1.Radiation Epidemiology Branch, Division of Cancer Epidemiology and GeneticsNational Cancer Institute, NIH, DHHSRockvilleUSA

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